Saturday, April 2, 2011

Walter's Story: Exercise & Cystic Fibrosis

Why am I 45 years old with Cystic Fibrosis? I was told I’d be lucky to see out my teens. Exercise has always played a big role in my life, and most likely is the reason why I am still in the land of the living.

My parents weren’t great sports fanatics, and I wasn’t much good at any sport. In fact I was always last to be chosen for any school team. I was the smallest in my class for most of my teens. I did love riding my bicycle to high school in Holland, 10kms each way! My parents could not get me to go in the bus, as I would always prefer to ride at high speed (with my neighbour) on the Dutch bicycle paths.

Once we immigrated to Australia, when I was 13, my family adopted an Irish Setter. I ran with it every day. In my late teens I also had a job as a milk-runner; delivering milk to peoples doorsteps from the back of a truck. I ran with ‘little athletics’, walked in the mountains with the FBI (Family Bushwalkers Incorporated), I did lots of outdoors activities with school such as hiking and caving.

Never was I very competitive, not ever did I need or want to come first in anything. My aim was to be outdoors and having fun. I don’t have a competitive bone in my body in fact. This also meant I didn’t have too many sport injuries in life. Even when I ran in marathons, half marathons and tri and duathlons I would only ever go in them for fun and just to finish them. I would cough so much during those events that people would regularly ask me if I was OK. My standard answer was; ‘Yes mate, I’ve coughed this distance before’. Swimming was the hardest for me. Maybe because in public pools it was not acceptable to cough and spit, so my training was limited there.

For me the exciting thing about exercise was variety. The more the better. I am easily bored, so I always tried new things. Loved roller blading, which I did with my mates on Sunday afternoons on an abandoned school play-ground. During the week, one of us bladers would sometimes find an adrenaline filled and mostly downhill course of 10-20 kms. When I was in my late twenties I beat the scuba diving medical test (by telling them I was a heavy smoker!) and started diving. After about 200 dives over maybe 5 years I realised how stupid I had been, or how lucky not to have had a pneumothorax! I was able to comfortably have huge coughing fits 20 meters under water, and even got my Advanced Open Water Padi certification. I still enjoy snorkelling, but when snorkelling I need a flotation device for when I cough, unlike when diving with a regulator in your mouth and a BCD (Buoyancy Control Device) keeping you steady.

Fun runs and community sporting events are always the most fun. Usually they have good prizes, and I often score an encouragement reward (and CF awareness!), or for having travelled the furthest to be there. And yes, sometimes I even end up first in my division (when I happen to be the only one in it!), like with Orienteering recently!

Last weekend I cycled in a big charity bicycle ride and I thought it was more of a social ride. Low and behold it was full of fancy gear road riders and I rode the entire distance on my own. I was the only one who brought lunch and snacks, and had a great ride on my own up the back.

Skiing is another great sport I loved doing. Although a little expensive now, I discovered that by doing cross country or trying out snow boards you don’t need the expensive full lift passes. I also discovered that if you rent a snow board they happily change it for cross country skis after lunch. When I was under 18 there were loads of affordable skiing offers for minors, complete with bus and lift tickets. I had rock-hoppers (old skis) that were so rough that I had no qualms about skiing down rocky patches, and got a ‘Wally of the Year’ award for skiing Thredbo from top to bottom when there was only half cover – with sparks. I had a lot of fun!

All through my life I have stayed relatively healthy mostly through sports and adventures, but of course my lungs have declined and are dipping towards the 40% of predicted FEV1. I still ride my bicycle everyday to and from my 2 hours a day desk-job, and try and do a daily ‘proper’ exercise (walk in the hills for an hour or so). I regularly take the kayak out for a paddle or go into the mountains, jog around the beach or play with the Wii inside. When I am lucky someone invites me to go sailing or mountain climbing, but mostly I do more accessible sports.

For work I have a casual office job where I do IT and Admin support for 2 hours a day in town, close enough to commute on the bicycle. I also do some freelance computer work which I can do from my laptop and I waste an awful lot of time doing just ‘stuff’ on the computer, mostly voluntary web work, blogs, and trying to write a second book. There is never enough time in my day as you can imagine, I also do a lot of volunteering where I can. A member of the local Lions group, and a Friend of the Lillico Penguins (a nearby colony of little penguins), help with bird counts and wherever people can use my help,...

And CF? Yes, that is a job on its own. Doing a thorough saline neb in the morning with huffs, puffs and flutter takes me a good hour, again at night with Pulmozyne too. My girlfriend ensures I never go hungry, feeding me day and night, and making sure I don’t forget anything (a huge job!), from Calcium tablets to insulin, reminding me to take my enzymes, my keys and my lunch. She comes with me on many of my exercises, including running with the Hash House Harriers (probably my most favourite exercise as it includes a beer or three), coming to Hobart to the CF Clinic, collecting for CF on 65 Roses day,... I think CF is team work, and we keep each other motivated. A while ago I took a cruise with my 77 year old mother (for her birthday), but even there I woke up at 6am every day to get to the ship’s gym for the onboard boot-camp classes, and on the island stops still managed to abseil down a waterfall... Never stop exercising!

I like to keep adventures on the horizon, like in the past when I rode across Europe (, or last year when I climbed Mount Kinabalu ( and next year I am hoping to do the El Camino trail, a 6-week walk across Spain.

Next time I might write about all the alternative medicine and therapies I have tried with varying results.

BIO: Name is Walter van Praag. Born in Holland 8 July 1965, diagnosed with CF at age 10, migrated to Australia at age 13. Educated in Canberra, complete with BA in Philosophy and BSc in Mathematics (not because I was smart, just because university life was perfectly suited to CF!). Ended up working with computers for a living after having gone through numerous government jobs and even set up and operated a small transport business! Worked in HK for a year, 3 years in PNG, 3 years in USA, travelled the world on a budget at every opportunity. Greatest claim to fame was receiving an Order of Australia Medal for 'service to community health through raising the awareness of cystic
fibrosis'. My personal biggest achievement was writing my first book; 'Coughing the Distance - cycling from Paris to Istanbul with CF'.

Note from Ronnie: Thank you SO MUCH Walter for letting us peak into your daily life of exercise and Cystic Fibrosis. You've provided us with great examples of different types of exercise we can be doing and ways that we can "keep it fresh". It's obvious that exercise has played a big role in your life and there is no doubt it is a huge reason that you are still kicking major booty today! Keep up the good work and thank you for all of your time and effort put forth into CF awareness.

Friday, April 1, 2011

First Friday: It's a Human...

...and it MOVES!!

Yesterday's ultrasound was by far the coolest yet. Our little peanut has graduated from looking like a seahorse, to looking like a real, live baby! We could instantly see little arm and leg buds and peanut actually has a visible head - in fact, a head that made up about 1/2 of the body (which we're told is normal, but scares me to death for the labor! If peanut has Ronnie's head, it could be very interesting!) Also very cool was the fact that this time you could see the umbilical cord, and watch the blood flow through it. It's little heart was beating even faster now - at about 178 beats a minute. The beating almost appeared in tandem with the blood flow through the umbilical cord.

And while all of that was so incredible to see, the most amazing part was that our baby was MOVING!! Yup, at just 9 weeks and 2 days, peanut was moving. We watched in amazement as it's whole body would wiggle, and as the little arms and legs would punch and kick. We're attaching a video, so you can see the wonder that is a tiny baby moving. You'll have to watch VERY closely though, because the movement isn't noticeable if you blink or aren't focused.

All in all, it was one of the most incredible experiences of my life - I think even more exciting than seeing the heartbeat for the first time. Something about peanut actually looking like a baby AND moving made it all so real.

Thursday, March 31, 2011

Thankful Thursday - Trial Runs & Ice

Know what time it is? It's thankful Thursday time! We all have so much to be thankful for and we love to take this opportunity just to write down each and everything that comes to mind. as I mentioned, I no longer have the "linky tools" but I invite you to share your thankful blog in the comments section. Without further ado, here's what we're thankful for:

Mandi's List:

I'm thankful that our infertility clinic gives away ultrasounds like candy (and they actually give out candy too!!) I am so very thankful that we have an ultrasound today, and I'm so thankful that we are able to frequently able to check up on peanut. The peace of mind that it gives me to see the little heart beat flutter is much needed.

I'm thankful for small group. We have been going to a small group - consisting of couples from our Sunday morning After I Do Sunday school class...made up of people who have been married less than 5 years. I always enjoy our Wednesday small group and leave feeling like I've learned something new to apply in my marriage, and feeling fulfilled after a night of fellowship.

I'm thankful for ice. Our ice maker was on the fritz the last few days. And our handy man (Ronnie) took time time yesterday to figure out the issue and got it fixed. Man, I didn't know how much I loved ice in my drinks before we didn't have it around. A freezing cold glass of water never tasted so good.

I'm thankful that I'm off of Progesterone shots. Not only is my bum starting to feel better, but I have felt slightly less tired since stopping the injections. I find I'm still sleepy at night, but the last two days I haven't been as tired mid-day, and I have been sleeping sounder at night. I am so thankful that I had the medicine to help our baby at the beginning of my pregnancy, and I'm even more thankful that my body has taken over and I'm done with the shots!

Ronnie's List:

I'm very thankful for our gym. A few days ago I hurt my foot running and haven't been able to lace up shoes and pound the pavement since then. Thankfully, our gym provides a ton of different exercise equipment that doesn't pound on my foot. A couple days ago I did the hand cycle and yesterday I did the exercise bike. Not only does it have a variety of exercise equipment, but they're all equipped with TVs which makes time fly by!

I'm thankful for our garden. Last year upon moving into this house the previous owners left us with a flourishing garden which I very quickly killed. I'm now a year older and wiser and looking forward to the opportunity to tend to my crops :) I'm not promising that everything will live, but I do promise to do better than last year!

I'm thankful for trial runs. Yesterday we had the opportunity to take care of my little cousin who is around two years old. Let me just say that if Mandi and I are lucky enough to have a kid half as good as Luke, we would be in heaven. We did some arts and crafts and then I cleaned the kitchen as Mandi and Luke fell asleep to SpongeBob SquarePants. It is just nice to experience having kids around even if it is just a little taste of what our life will be like in a couple of years.

So, what are you thankful for today?

Wednesday, March 30, 2011

Rondi's Recipes: Irish Corned Beef Stew

In honor of St. Patty's day a little while back, Mandi and I decided to make some Irish Stew. We didn't follow the recipe to a tee, but we were close enough not to call it our own creation :) If you want to see the original recipe, please click here.

Corned Beef

Potatoes, Carrots and Parsnip...oh my!

Guinness beer...pretty sure we have 5 bottles left.

Chopped Onions

Looks better than I remember

Enjoy...with enzymes on the side of course!

Prep Time: About 20 minutes (with two people)
Cook Time: About 3 hours
Total Time: About 3 hours and 30 minutes

What we liked:

I really like soups/stews that are chunky. This stew definitely fit the bill.
I'm a fan of any hot soup/stew during a cold night.
I thought it was really easy to make.
Cleanup was easy. One cutting board. One pot. One knife.

This meal was very hearty! I tend to love chunky soups more than your average bear.
It was a little chilly out and I don't think it can get much better as far as a "comfort" stew goes.
Meat and potatoes. I could probably eat that everyday of the week. Winning.
The prep was very easy and once it started to cook, really didn't have to do much.
We also added cabbage to the stew, another thing that I could eat everyday (stewed).
I love corned beef.

What we'd change:

I thought it could be more flavorful. I don't know what I'd change, but I'd add something next time.
Speaking of flavor, it might be better if we seasoned the meat or even let it soak in a marinade.
The pieces of cabbage were a little big for my liking. Next time they need to be smaller.
I want to try this out in the slow cooker.

I had to add quite a bit of salt to this meal to maximize the flavor.
The meat chunks were actually a little too big.
I'd probably want to add another vegetable or two.
I'd also like to try a similar recipe in the slow cooker.

Here's our overall rating (with 1 being the worst meal we've ever had and 10 being the best):

Mandi's Rating: 7

Ronnie's Rating: 6.0

Again, if you guys have any suggestions on how this is laid out or what you guys would like to see answered/done differently, please let us know :)

Tuesday, March 29, 2011

Graduating to an OBGYN

Yup, graduating. At least that's what it felt like when I walked into my OBGYN's office yesterday morning. I had a strange sense of pride as I filled out my forms. I somehow felt like I now had a "normal" pregnancy. I now was at an appointment that everyone goes to. It felt good. It felt like we had crossed a certain threshold; like we made it to a certain point; like we were graduating onto "pregnancy" and not "trying to get pregnant." Unfortunately this graduation isn't as cut and dry as high school to college or college to the work force. We still have a little bit of overlap, as our fertility doc wants to see us another few weeks, but it feels good to be starting the transition.

Yesterday's appointment was just a meet and greet. I am going to a new OBGYN, so I had to fill out some new patient paperwork and then just sat down and chatted with a PA. She was oober nice. She felt like my BFF (yes, I did just use "BFF) and even hugged me as she headed out of the office. We'll be headed back next Monday for our first ultrasound with them, and then they'll see us every four until it's gets closer to D day.

Yesterday was also my LAST DAY OF PROGESTERONE INJECTIONS! If you can't tell, I'm flippin' excited - and so is my incredibly bruised, sore bum. I am thrilled that I am done with the shots, but a little nervous too. You stop the injections when your body takes over. And I'm just hoping my body will take over as it should. There's absolutely no reason it shouldn't, wouldn't and couldn't, but a worrier will still worry :) Supposedly I will feel way better once I'm off the Progesterone. They told me at my appointment yesterday that it makes you more tired, more bloated, and more "down". The only catch is that the Progesterone can keep morning sickness at bay (I learned for the first time yesterday). So I could be in for a little reality check in the next week or so. Only time will tell!

Thursday we head to see our fertility docs for another ultrasound. Again just totally routine and more for our enjoyment than their need. I cannot wait to see little peanut's heartbeat and what it's looking like this week. Hopefully less like a sea creature and more like a human! This week, according to, our baby is the size of a grape. It has arms and legs, even earlobes - I wonder if its earlobes will be as soft as its Daddy's?

That's it! We'll post pictures of my belly next week, and ultrasound pictures on Friday. Any guesses what it will look like this week? Maybe a Platypus?

Monday, March 28, 2011

Understanding Cystic Fibrosis: Biofilm

There was a recent conversation on CysticLife regarding sputum cultures and their effectiveness in determining the right course of antibiotics. This conversation then led into a short discussion about biofilm which got me thinking that many of us probably aren't that familiar with what it is and what it has to do with Cystic Fibrosis. In my quest for all of us to understand this disease a little bit better, I wanted to post the abstract and conclusion from a recent research article that I read regarding biofilm. Enjoy!

You can find the full article on or by clicking here (you must sign up for a free account to view the entire article)


The persistence of chronic Pseudomonas aeruginosa lung infections in cystic fibrosis (CF) patients is due to biofilm-growing mucoid (alginate-producing) strains. A biofilm is a structured consortium of bacteria, embedded in a self-produced polymer matrix consisting of polysaccharide, protein and DNA. In CF lungs, the polysaccharide alginate is the major part of the P. aeruginosa biofilm matrix. Bacterial biofilms cause chronic infections because they show increased tolerance to antibiotics and resist phagocytosis, as well as other components of the innate and the adaptive immune system. As a consequence, a pronounced antibody response develops, leading to immune complex-mediated chronic inflammation, dominated by polymorphonuclear leukocytes. The chronic inflammation is the major cause of the lung tissue damage in CF. Biofilm growth in CF lungs is associated with an increased frequency of mutations, slow growth and adaptation of the bacteria to the conditions in the lungs, and to antibiotic therapy. Low bacterial metabolic activity and increase of doubling times of the bacterial cells in CF lungs are responsible for some of the tolerance to antibiotics. Conventional resistance mechanisms, such as chromosomal β-lactamase, upregulated efflux pumps, and mutations of antibiotic target molecules in the bacteria, also contribute to the survival of P. aeruginosa biofilms. Biofilms can be prevented by early aggressive antibiotic prophylaxis or therapy, and they can be treated by chronic suppressive therapy.

Conclusion & Future Perspective

Pseudomonas aeruginosa adapts to the respiratory zone and to the innate and adaptive defense mechanisms of the lungs by forming mucoid biofilms, which survive for decades, in spite of the inflammatory response, where the lung tissue is gradually destroyed. P. aeruginosa also adapts to intense antibiotic therapy by forming mucoid biofilms, but also by conventional resistance mechanisms, which act synergistically to allow P. aeruginosa to survive. P. aeruginosa adapts to the anaerobic conditions in sputum in the conductive zone of the lungs by splitting-off nonmucoid variants, which do not form biofilm in CF patients and, probably, do not play any major clinical role. Biofilm formation is, therefore, the major survival mechanism of P. aeruginosa in the lungs of CF patients, and it is the clinically most important mechanism.

A promising new strategy may be to target biofilm formation by the use of enzymes and other chemicals, such as polyvalent anions, which can dissolve the biofilm matrix (e.g., DNase and alginate lyase). Also, QS inhibitors increase biofilm susceptibility to antibiotics. Induction of neutralizing antibodies against chromosomal β-lactamase of P. aeruginosa improves the clinical outcome of antibiotic therapy with β-lactam antibiotics.This strategy, therefore, may be utilized clinically in the future to neutralize one of the important conventional resistance mechanisms. Possible prophylactic measures could also be use of anti-inflammatory drugs, or antioxidant therapy to prevent ROS-induced mutations in the mucA gene, or use of macrolides, such as azithromycin, in doses that inhibit QS and alginate synthesis, as suggested by in vitroresults and animal experiments.